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Pancreatic PEComa, a not so uncommon neoplasm? Systematic review and therapeutic update.

Pancreatic PEComa, a not so uncommon neoplasm? Systematic review and therapeutic update.

Authors :
Nogueira Sixto M
Carracedo Iglesias R
Estévez Fernández S
Rodríguez Pereira C
Sánchez Santos R
Source :
Gastroenterologia y hepatologia [Gastroenterol Hepatol] 2024 Jan; Vol. 47 (1), pp. 93-100. Date of Electronic Publication: 2023 May 24.
Publication Year :
2024

Abstract

Pancreatic PEComas are extremely rare neoplasms with malignant potential, which mostly affect middle-aged women and are characterized by presenting melanocytic and myogenic markers in immunohistochemical analysis. There are no symptoms or pathognomonic imaging tests, so the diagnosis is established with the analysis of the surgical specimen or the FNA obtained with preoperative endoscopic ultrasound. The mean treatment consists on radical excision, adapting the intervention to the location of the tumor. To date, 34 cases have been described; however, more than 80% of them have been reported in the last decade, which suggests that it is a more frequent pathology than expected. A new case of pancreatic PEComa is reported and a systematic review of the literature is carried out according to the PRISMA guidelines with the aim of divulge this pathology, deepening its knowledge and updating its management.<br /> (Copyright © 2023 Elsevier España, S.L.U. All rights reserved.)

Details

Language :
English; Spanish; Castilian
ISSN :
0210-5705
Volume :
47
Issue :
1
Database :
MEDLINE
Journal :
Gastroenterologia y hepatologia
Publication Type :
Academic Journal
Accession number :
37230381
Full Text :
https://doi.org/10.1016/j.gastrohep.2023.05.009